Indeed, comparison of haematoxylin-eosin (HE) and elastin stained aortas of 3-month old Fibulin-4+/+, Fibulin-4+/R and Fibulin-4R/R mice showed severe thickening and decellularization of the medial layer of the ascending aortic wall in Fibulin-4R/R mice and fragmented and disorganized elastin laminae resulting in 2–3 fold dilated ascending aortic aneurysms in all homozygous knockdown animals analyzed (Figure 2A). The gene discussed is EFEMP2; the disease is Ascending tubular aorta aneurysm.